scholarly journals Surgical Management of Hearts with Isomeric Atrial Appendages

2022 ◽  
Author(s):  
Ujjwal Chowdhury ◽  
Robert Anderson ◽  
Diane E. Spicer ◽  
Lakshmi Sankhyan ◽  
Niraj Pandey ◽  
...  
Keyword(s):  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Double Outlet Right Ventricle ◽  
Surgical Approaches ◽  
Caval Vein ◽  
Inferior Caval Vein ◽  
Left Isomerism ◽  
Right Isomerism ◽  
Pulmonary Arterial ◽  
Anomalous Pulmonary Venous Connection

Background and aim: On the basis of previously published accounts, coupled with our own experience, we have assessed the surgical approaches to patients with isomeric atrial appendages. Methods: We reviewed pertinent published studies on surgical treatment of individuals with isomeric atrial appendages, with the pertinent surgical details provided by most of the manuscripts. Results: Half of patients with right isomerism, and two-thirds of those with left isomerism have bilateral superior caval veins. Azygos extension of the inferior caval vein is reported in three-quarters of those with left isomerism. The coronary sinus is universally absent in right isomerism, along with totally anomalous pulmonary venous connection, and is absent in two-fifths of those with left isomerism.. Univentricular atrioventricular connections are expected in up to three-quarters of those with right isomerism. Atrioventricular septal defect is reported in up to four-fifths, more frequently in right isomerism, with such patients typically having discordant ventriculoatrial connections or double outlet right ventricle. Reported mortalities extend to 85% for those with right, and 50% for those with left isomerism. In right isomerism, mortality is up to 54% for systemic-to-pulmonary arterial shunting, up to 75% for univentricular repair, and up to 95% for repair of totally anomalous pulmonary venous connection itself. No more than one-quarter had undergone Fontan completion, with reported mortalities of 21%. Conclusion: Early surgical results are satisfactory in patients with left isomerism, but disappointing for those with right. Recent advances in cardiac and liver transplantation may offer improved survival.

Segregating bodily isomerism or heterotaxy: potential echocardiographic correlations of morphological findings

2017 ◽  
Vol 27 (8) ◽  
pp. 1470-1480 ◽  
Author(s):  
Cornelia Tremblay ◽  
Rohit S. Loomba ◽  
Peter C. Frommelt ◽  
Donald Perrin ◽  
Diane E. Spicer ◽  
...  
Keyword(s):  
Coronary Sinus ◽  
Venous Return ◽  
Caval Vein ◽  
Segmental Analysis ◽  
Morphological Findings ◽  
Inferior Caval Vein ◽  
Left Isomerism ◽  
Right Isomerism ◽  
Systemic Venous Return ◽  
Anomalous Pulmonary Venous Connection

AbstractBackgroundBodily isomerism, also referred to as heterotaxy, involves predominantly the thoracic organs, although other organs are usually abnormally positioned. Previously assessed on the basis of splenic anatomy, it is now understood that isomerism is better segregated on the basis of atrial appendage morphology. This allows for anticipation of associated findings. We aimed to assess the accuracy of segregation based on the morphology of the atrial appendages and other structures more easily identified by echocardiography.MethodsWe reviewed postmortem specimens of hearts from the archives at four institutions categorised as obtained from patients with “heterotaxy”. The cardiac structures were analysed using sequential segmental analysis. Non-cardiac structures were also examined if available. Statistical analyses were performed to compare differences in the settings of right as opposed to left isomerism.ResultsSpecimens were available from 188 patients. Of these, 57 had left isomerism, and 131 had right isomerism. Atrial appendages were isomeric in all patients. A coronary sinus was found only in left isomerism, whereas a terminal crest, or a Eustachian valve, was found only in right isomerism. Interruption of the inferior caval vein was associated with left isomerism, whereas totally anomalous pulmonary venous connection was associated with right isomerism.ConclusionIsomerism is uniformly segregated on the basis of the morphology of the atrial appendages, itself defined by the extent of the pectinate muscles. Other features such as the presence of a coronary sinus and systemic venous return can further help with such segregation of isomerism.

Left-sided hepatic vein connected to the coronary sinus

1996 ◽  
Vol 6 (2) ◽  
pp. 190-192 ◽  
Author(s):  
László Király ◽  
John E. Deanfield ◽  
Marc R. de Leval
Keyword(s):  
Hepatic Vein ◽  
Coronary Sinus ◽  
Left Atrial ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Rare Entity ◽  
Biventricular Repair ◽  
Caval Vein ◽  
Inferior Caval Vein ◽  
Complete Atrioventricular

AbstractA left-sided hepatic vein connected to the coronary sinus is reported in a case of a 22-month-old boy with isomerism of the left atrial appendages, complete atrioventricular septal defect and azygous continuation of the inferior caval vein. The diagnosis of the anomalous hepatic vein was made intraoperatively and successful biventricular repair has been accomplished. To the best of our knowledge, this is the first communication on this peculiar entity diagnosed during life, notwithstanding Nabarro's description of a similar autopsy finding in 1903. Aspects of the development of this rare entity are discussed.

Implantation of drug-eluting stents for relief of obstructed infra-cardiac totally anomalous pulmonary venous connection in isomerism of the right atrial appendages

2008 ◽  
Vol 18 (6) ◽  
pp. 628-630 ◽  
Author(s):  
Derek T. H. Wong ◽  
Shi-Joon Yoo ◽  
Kyong-Jin Lee
Keyword(s):  
Septal Defect ◽  
Pulmonary Atresia ◽  
Atrioventricular Septal Defect ◽  
Right Atrial ◽  
Collateral Arteries ◽  
Vertical Vein ◽  
Right Isomerism ◽  
Drug Eluting ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

AbstractWe describe an infant with severe obstruction of infra-cardiac totally anomalous pulmonary venous connection associated with right isomerism, atrioventricular septal defect, pulmonary atresia, and multiple aortopulmonary collateral arteries. Implantation of a stent into the obstructed descending vertical vein provided effective palliation, with a dramatic increase in saturations of oxygen obviating the need for urgent high-risk surgery.

Characteristics of the pulmonary circulation in infants with complete atrioventricular septal defect

2020 ◽  
pp. 1-6
Author(s):  
Hirohito Doi ◽  
Jun Muneuchi ◽  
Mamie Watanabe ◽  
Yuichiro Sugitani ◽  
Ryohei Matsuoka ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Gestational Age ◽  
Pulmonary Circulation ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Left Heart ◽  
Complete Atrioventricular Septal Defect ◽  
Pulmonary Arterial ◽  
Heart Lesions ◽  
Complete Atrioventricular

Abstract Objective: Infants with complete atrioventricular septal defect occasionally accompany pulmonary hypertension; however, the pulmonary circulation can be altered by pulmonary vascular conditions as well as the left heart lesions. This study aimed to explore whether the left heart lesions were related to the pulmonary circulation among them. Methods: We performed echocardiography and cardiac catheterisation in 42 infants with complete atrioventricular septal defect and studied relationships between the pulmonary haemodynamic parameters and the left heart morphology. Results: Age and weight at preoperative evaluation were 65 days (47-114) (the median following interquartile range) and 5.5 kg (4.0-7.1), respectively. There were 27 individuals with Down syndrome. Gestational age was 38 weeks (37-39). Catheterisation showed mean pulmonary arterial pressure: 36 (29-46) mmHg, the ratio of pulmonary to systemic blood flow: 3.45 (2.79-4.98), pulmonary vascular resistance: 2.20 Wood units·m2 (1.53-3.65), and pulmonary arterial compliance: 2.78 (1.86-4.10) ml/Hg/m2. Echocardiography showed the Rastelli classification type A in 28 and type C in 14, moderate or severe left atrioventricular valve regurgitation in 19 patients (45%), atrioventricular valve index of 0.67 (0.56-0.79), left ventricular end-diastolic volume z score of 4.46 (1.96-7.78), and aortic valve diameter z score of −0.70 (−1.91 to 0.20). Multivariable regression analysis revealed that preoperative pulmonary vascular resistance was significantly correlated to gestational age (p = 0.002), and that preoperative pulmonary arterial compliance was significantly correlated to gestational age (p = 0.009) and Down syndrome (p = 0.036). Conclusions: The pulmonary circulation does not depend upon the presence of left heart lesions but gestational age and Down syndrome in infants with complete atrioventricular septal defect.

Hepatic venous connection to a persistent inferior caval vein in left isomerism

1990 ◽  
Vol 11 (9) ◽  
pp. 845-847 ◽  
Author(s):  
J. GUENTHARD ◽  
J. S. CARVALHO ◽  
R. H. ANDERSON ◽  
M. L. RlGBY
Keyword(s):  
Caval Vein ◽  
Inferior Caval Vein ◽  
Left Isomerism

Totally anomalous pulmonary venous connection through the right lung and via a “Scimitar” vein to the inferior caval vein

1993 ◽  
Vol 3 (1) ◽  
pp. 85-87
Author(s):  
Rakesh Dua ◽  
Christine McTigue ◽  
James.L Wilkinson
Keyword(s):  
Pulmonary Vein ◽  
Pulmonary Veins ◽  
Left Lung ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Caval Vein ◽  
Inferior Caval Vein ◽  
The Right ◽  
Left Heart Syndrome ◽  
Anomalous Pulmonary Venous Connection

AbstractWe report a case of totally anomalous pulmonary venous connection in which the two pulmonary veins from the left lung joined to form a common vein which then passed across the midline into a hypoplastic right lung and, after receiving small veins from the right lung, passed inferiorly, exiting the lung below the hilum as a “scimitar” vein and terminating in the inferior caval vein. A separate pulmonary vein from the right lung passed inferiorly independently and joined the “scimitar” vein before it entered the inferior caval vein. There was an associated hypoplastic left heart syndrome.

Horseshoe lung associated with anomalous pulmonary venous connection without pulmonary hypoplasia

1995 ◽  
Vol 5 (1) ◽  
pp. 91-93 ◽  
Author(s):  
Antonio Corno ◽  
Luca Rosti ◽  
Ivan Machado
Keyword(s):  
Ventricular Septal Defect ◽  
Congenital Malformation ◽  
Septal Defect ◽  
Pulmonary Hypoplasia ◽  
Caval Vein ◽  
Superior Caval Vein ◽  
Horseshoe Lung ◽  
Anomalous Pulmonary Venous Connection

SummaryHorseshoe lung is an exceedingly rare congenital malformation, characterized by unilateral pulmonary hypoplasia together with a midline isthmus producing fusion of the tissues of the lower lobes. It is frequently associated with other cardiac and extracardiac anomalies. We report an infant with a variant of the horseshoe lung with partial anomalous venous connection of intracardiac type, ventricular septal defect, and persistent left superior caval vein. There was no pulmonary hypoplasia.

Anatomic biventricular repair by intraatrial and intraventricular re-routing in patients with left isomerism

2001 ◽  
Vol 11 (1) ◽  
pp. 12-16 ◽  
Author(s):  
Hideki Uemura ◽  
Toshikatsu Yagihara ◽  
Youichi Kawahira ◽  
Yoshiro Yoshikawa
Keyword(s):  
Left Atrial ◽  
Right Ventricular Outflow Tract ◽  
Functional Results ◽  
Ventricular Outflow Tract ◽  
The Other ◽  
P Wave ◽  
Biventricular Repair ◽  
Caval Vein ◽  
Inferior Caval Vein ◽  
Left Isomerism

AbstractObjective: To determine the efficacy of anatomic biventricular repair by a combination of intraatrial and intraventricular re-routing in patients with isomerism of the left atrial appendages.Methods: Anatomic biventricular repair by means of combined intraratrial and intraventricular re-routing was achieved in 5 of the 63 patients with left isomerism in whom we attempted a definitive surgical procedure. The inferior caval vein was interrupted in 3. The Mustard procedure was chosen for intraatrial redirection of blood in 4, and the Senning procedure in the other. Intraventricular re-routing was carried out via a right ventriculotomy in all patients, using an external conduit to reconstruct the morphologically right ventricular outflow tract.Results: One patient died one month after the procedure because of low cardiac output and bronchial bleeding. Obstruction across the superior caval venous channel occurred after the Senning procedure in this particular patient, related to the interrupted inferior caval vein draining via the azygous vein. The other 4 patients are currently doing well. Postoperative catheterization showed excellent cardiac performance, with no obstruction across the venous channels or the ventricular outflow tracts in these 4. No episode of significant archythmia has been noted, all patients having a regular atrial rhythm, although the P wave vector was unusual in each patient. Reoperation has been needed thus far in one patient, 128 months after the initial repair, because of obstruction of the external conduit.Conclusion: With precise recognition of the morphologic features, a combination of intraatrial and intraventricular re-routing can successfully be established in patients with isomeric left atrial appendages, with functional results in the intermediate term being reasonable.

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